Nephrotic Range Proteinuria due to ANCA-Associated Vasculitis in a Diffuse Systemic Sclerosis Patient: A Rare Presentation
Bibliographic record
Abstract
Introduction: Scleroderma renal crisis (SRC) is a severe complication of SSc and typically presents with new-onset hypertension and a reduction in renal functioning. Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a rare co-occurrence with SSc. We present a rare case of diffuse systemic sclerosis who presented with features of SRC and AAV with nephrotic range proteinuria without significant renal failure. Case Description: The patient is a 32-year old woman with diffuse systemic sclerosis complicated by pulmonary fibrosis who had been treated with Mycophenolate mofetil but she discontinued treatment in order to conceive. She reports having an uncomplicated pregnancy without proteinuria or hypertension, but she delivered 7 weeks early. After delivery, she began experiencing weakness of her lower extremities and given steroids for suspecting demyelinating neuropathy and restarted treatment with Mycophenolate mofetil. She had improved but few weeks later (3 months after delivery), she was readmitted with hypertensive encephalopathy in setting of suspected scleroderma renal crisis. She also developed a rash and swelling in her legs after her pregnancy. Her urinalysis was remarkable for proteinuria and numerous RBCs and quantification of the urine protein revealed nephrotic range proteinuria (4.6 g/24 hours) but her serum creatinine (0.6 mg/dl) remained normal. She was found to be positive for antinuclear antibodies, rheumatoid factor, myeloperoxidase antibodies, MPO-ANCA (> 8.0 IU/mL). Her complement levels were within normal limits. She underwent a renal biopsy, which revealed an acute necrotizing vasculitis consistent with AAV. Discussion: Clinicians should remain vigilant for concomitant autoimmune disorders in patients with scleroderma. In the background of systemic sclerosis, our patient developed AAV with neurologic and renal manifestations. Thus, renal injury, proteinuria or rapid onset of hypertension should not be assumed to be due to scleroderma renal crisis, particularly when there is nephrotic range proteinuria or an active urine sediment.
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.000 | 0.002 |
| Meta-epidemiology (narrow) | 0.002 | 0.001 |
| Meta-epidemiology (broad) | 0.001 | 0.001 |
| Bibliometrics | 0.002 | 0.001 |
| Science and technology studies | 0.002 | 0.001 |
| Scholarly communication | 0.001 | 0.001 |
| Open science | 0.001 | 0.001 |
| Research integrity | 0.003 | 0.002 |
| Insufficient payload (model declined to judge) | 0.003 | 0.001 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".